Chapter 3. Amyotrophic Lateral Sclerosis

  1. Prof. Dr. Mathias Bähr
  1. Georg Haase

Published Online: 17 JUN 2005

DOI: 10.1002/3527603867.ch3

Neuroprotection: Models, Mechanisms and Therapies

Neuroprotection: Models, Mechanisms and Therapies

How to Cite

Haase, G. (2004) Amyotrophic Lateral Sclerosis, in Neuroprotection: Models, Mechanisms and Therapies (ed M. Bähr), Wiley-VCH Verlag GmbH & Co. KGaA, Weinheim, FRG. doi: 10.1002/3527603867.ch3

Editor Information

  1. Department of Neurology, University of Göttingen Medical School, Robert-Koch-Str. 40, 37075 Göttingen, Germany

Author Information

  1. Institut de Neurobiologie de la Mediterranée, Campus de Luminy, F-13288 Marseille Cedex 09, France

Publication History

  1. Published Online: 17 JUN 2005
  2. Published Print: 27 JUL 2004

ISBN Information

Print ISBN: 9783527308163

Online ISBN: 9783527603862



  • neuroprotection models;
  • mechanisms and therapies;
  • neurological disorders – epidemiology;
  • clinical overview;
  • model systems;
  • amyotrophic lateral sclerosis;
  • human motor neuron diseases;
  • cell culture models of motoneuron degeneration;
  • animal models of motor neuron disease;
  • mutant SOD1 mice;
  • future neuroprotective approaches


This chapter contains sections titled:

  • Human Motor Neuron Diseases

    • Familial ALS Linked to SOD1 Mutations

    • Juvenile ALS Linked to Alsin Mutations

  • Cell Culture Models of Motoneuron Degeneration

    • Neuronal Cell Lines

    • Isolated Motoneuron Cultures

    • Organotypic Cultures

  • Animal Models of Motor Neuron Disease

    • Axotomy Models

    • Mutant SOD1 Mice

    • pmn Mice

  • Future Neuroprotective Approaches

    • Axonal Protection

    • Mutant SOD1 Knockdown by RNA Interference

  • Conclusion

  • References